Epidermolysis bullosa simplex (intraepidermal epidermolysis bullosa) and allied conditions

Thirty years before the rediscovery of Mendel’s laws of inheritance intraepidermal non-scarring blistering was called Erblichen Pemphigus by von Hebra [1]. Kobner in 1886 called the same condition epidermolysis bullosa hereditaria (EBH) [2]. Since the latter term soon became adopted for several congenital traumatic blistering diseases, Hallopeau [3] found it necessary to call the original type’ simple’, i. e. epidermolysis bullosa simplex (EBS). By 1908 EBS was recognized as a dominant Mendelian trait [4]. Incidentally, Kobner’s cases had mainly blisters of the hands and feet with only occasional blisters elsewhere. Many similar families were later called localized EBS (Weber-Cockayne) named after Weber’s (1926) solitary ‘recurrent bullous eruption of the feet’ case [5] and Cockayne’s (1938) two dominant families with blistering feet only [6]. Such cases had previously [7] been called EBS, but Cockayne distinguished them from EBS. Since 1957 [8], EBS (Weber-Cockayne) has been used for cases of blistering of the feet or hands and feet, and EBS (Kobner) for more generalized blistering. One of Cockayne’s families had the additional feature of ‘easily bruising skin’ and may therefore represent a different disease (EBS Ogna) first distinguished in 1970 [9].